- Pediatric Critical Care Medicine
Clinical Associate Professor, Pediatrics - Critical Care
Program Director, Pediatric Critical Care Fellowship (2019 - Present)
Associate Program Director, Pediatric Residency Program (2017 - Present)
Associate Program Director, Pediatric Critical Care Fellowship Program (2017 - 2019)
Rotation Director, Pediatric Intensive Care Unit (2017 - Present)
Honors & Awards
Teacher of the Year, Pediatric Critical Care Medicine Fellowship Program, Stanford University (2018)
Golden Apple Teaching Award-Selected by Pediatric Residents as Faculty Teacher of the Year, Department of Pediatrics, Stanford University (2018)
Boards, Advisory Committees, Professional Organizations
Faculty, University of Michigan Masters of Health Professions Education Program (2018 - Present)
Distinguished Member, Stanford University Teaching and Mentoring Academy (2019 - Present)
Member, (EPIC) Educators in Pediatric Intensive Care (2017 - Present)
Member, Pediatric Acute Lung Injury and Sepsis Investigators (PALISI) (2011 - Present)
Member, Society of Critical Care Medicine (2011 - Present)
Member, American Academy of Pediatrics (2008 - Present)
Residency: Stanford Health Care at Lucile Packard Children's Hospital (2011) CA
Medical Education: Harvard Medical School (2008) MA
Fellowship: University of Michigan Health System (2014) MI
MHPE, University of Michigan, Ann Arbor, Master's in Health Professions Education (2017)
Board Certification: American Board of Pediatrics, Pediatrics (2011)
Board Certification: American Board of Pediatrics, Pediatric Critical Care Medicine (2014)
Graduate and Fellowship Programs
Pediatric Critical Care Medicine (Fellowship Program)
Impact of a relationship-centered care communication curriculum on pediatric residents' practice, perspectives, and opportunities to evelop expertise.
Patient education and counseling
OBJECTIVES: To investigate the impacts of a Relationship-Centered Care (RCC) communication curriculum with coaching on pediatric residents 1) self-reported use of RCC strategies and perspectives, and 2) opportunities to develop adaptive expertise.METHODS: Residents (n=77) completed a 4h RCC training and shared resultant RCC goals with Coaches (n=15). Data included resident surveys and reflections immediately post-training, and resident and coach surveys 6-months later. Reported use of RCC strategies were compared over time with paired t-tests. Qualitative data were analyzed using open coding guided by sensitizing principles from the RCC framework and adaptive expertise.RESULTS: Pediatric residents reported significant increases (p<0.001) in use of 4/9 RCC strategies after 6 months: eliciting all concerns, chunking information, checking for understanding, and teach-back. Resident reflections highlighted shifts in perspective around RCC. Training combined with coaching provided opportunities for residents to develop adaptive expertise through adapting and innovating across settings and contexts.CONCLUSION: Residents had significant increases in reported use of key RCC strategies after a training combined with coaching and demonstrated opportunities to develop adaptive expertise.PRACTICE IMPLICATIONS: Residency programs should include RCC training with an emphasis on the new and challenging strategies and provide opportunities to practice and receive coaching.
View details for DOI 10.1016/j.pec.2021.08.014
View details for PubMedID 34538683
Impact of the COVID-19 Pandemic on the Clinical Learning Environment: Addressing Identified Gaps and Seizing Opportunities.
Academic medicine : journal of the Association of American Medical Colleges
2021; 96 (9): 1276-1281
The clinical learning environment (CLE) encompasses the learner's personal characteristics and experiences, social relationships, organizational culture, and the institution's physical and virtual infrastructure. During the COVID-19 pandemic, all 4 of these parts of the CLE have undergone a massive and rapid disruption. Personal and social communications have been limited to virtual interactions or shifted to unfamiliar clinical spaces because of redeployment. Rapid changes to the organizational culture required prompt adaptations from learners and educators in their complex organizational systems yet caused increased confusion and anxiety among them. A traditional reliance on a physical infrastructure for classical educational practices in the CLE was challenged when all institutions had to undergo a major transition to a virtual learning environment. However, disruptions spurred exciting innovations in the CLE. An entire cohort of physicians and learners underwent swift adjustments in their personal and professional development and identity as they rose to meet the clinical and educational challenges they faced due to COVID-19. Social networks and collaborations were expanded beyond traditional institutional walls and previously held international boundaries within multiple specialties. Specific aspects of the organizational and educational culture, including epidemiology, public health, and medical ethics, were brought to the forefront in health professions education, while the physical learning environment underwent a rapid transition to a virtual learning space. As health professions education continues in the era of COVID-19 and into a new era, educators must take advantage of these dynamic systems to identify additional gaps and implement meaningful change. In this article, health professions educators and learners from multiple institutions and specialties discuss the gaps and weaknesses exposed, opportunities revealed, and strategies developed for optimizing the CLE in the post-COVID-19 world.
View details for DOI 10.1097/ACM.0000000000004013
View details for PubMedID 34432665
Assessing Competence in Central Venous Catheter Placement by Pediatric Critical Care Fellows: A National Survey Study
CRITICAL CARE MEDICINE
2019; 47 (8): E654–E661
To describe the current approach to initial training, ongoing skill maintenance, and assessment of competence in central venous catheter placement by pediatric critical care medicine fellows, a subset of trainees in whom this skill is required.Cross-sectional internet-based survey with deliberate sampling.United States pediatric critical care medicine fellowship programs.Pediatric critical care medicine program directors of Accreditation Council for Graduate Medical Education-accredited fellowship programs.None.A working group of the Education in Pediatric Intensive Care Investigators research collaborative conducted a national study to assess the degree of standardization of training and competence assessment of central venous catheter placement across pediatric critical care medicine fellowship programs. After piloting, the survey was sent to all program directors (n = 67) of Accreditation Council for Graduate Medical Education-accredited pediatric critical care medicine programs between July 2017 and September 2017. The response rate was 85% (57/67). Although 98% of programs provide formalized central venous catheter placement training for first-year fellows, only 42% of programs provide ongoing maintenance training as part of fellowship. Over half (55%) of programs use a global assessment tool and 33% use a checklist-based tool when evaluating fellow central venous catheter placement competence under direct supervision. Only two programs (4%) currently use an assessment tool previously published and validated by the Education in Pediatric Intensive Care group. A majority (82%) of responding program directors believe that a standardized approach to assessment of central venous catheter competency across programs is important.Despite national mandates for skill competence by many accrediting bodies, no standardized system currently exists across programs for assessing central venous catheter placement. Most pediatric critical care medicine programs use a global assessment and decisions around the ability of a fellow to place a central venous catheter under indirect supervision are largely based upon subjective assessment of performance. Further investigation is needed to determine if this finding is consistent in other specialties/subspecialties, if utilization of standardized assessment methods can improve program directors' abilities to ensure trainee competence in central venous catheter insertion in the setting of variable training approaches, and if these findings are consistent with other procedures across critical care medicine training programs, adult and pediatric.
View details for DOI 10.1097/CCM.0000000000003821
View details for Web of Science ID 000475675500006
View details for PubMedID 31135502
Effect of Fresh vs Standard-issue Red Blood Cell Transfusions on Multiple Organ Dysfunction Syndrome in Critically Ill Pediatric Patients: A Randomized Clinical Trial.
2019; 322 (22): 2179–90
The clinical consequences of red blood cell storage age for critically ill pediatric patients have not been examined in a large, randomized clinical trial.To determine if the transfusion of fresh red blood cells (stored ≤7 days) reduced new or progressive multiple organ dysfunction syndrome compared with the use of standard-issue red blood cells in critically ill children.The Age of Transfused Blood in Critically-Ill Children trial was an international, multicenter, blinded, randomized clinical trial, performed between February 2014 and November 2018 in 50 tertiary care centers. Pediatric patients between the ages of 3 days and 16 years were eligible if the first red blood cell transfusion was administered within 7 days of intensive care unit admission. A total of 15 568 patients were screened, and 13 308 were excluded.Patients were randomized to receive either fresh or standard-issue red blood cells. A total of 1538 patients were randomized with 768 patients in the fresh red blood cell group and 770 in the standard-issue group.The primary outcome measure was new or progressive multiple organ dysfunction syndrome, measured for 28 days or to discharge or death.Among 1538 patients who were randomized, 1461 patients (95%) were included in the primary analysis (median age, 1.8 years; 47.3% girls), in which there were 728 patients randomized to the fresh red blood cell group and 733 to the standard-issue group. The median storage duration was 5 days (interquartile range [IQR], 4-6 days) in the fresh group vs 18 days (IQR, 12-25 days) in the standard-issue group (P < .001). There were no significant differences in new or progressive multiple organ dysfunction syndrome between fresh (147 of 728 [20.2%]) and standard-issue red blood cell groups (133 of 732 [18.2%]), with an unadjusted absolute risk difference of 2.0% (95% CI, -2.0% to 6.1%; P = .33). The prevalence of sepsis was 25.8% (160 of 619) in the fresh group and 25.3% (154 of 608) in the standard-issue group. The prevalence of acute respiratory distress syndrome was 6.6% (41 of 619) in the fresh group and 4.8% (29 of 608) in the standard-issue group. Intensive care unit mortality was 4.5% (33 of 728) in the fresh group vs 3.5 % (26 of 732) in the standard-issue group (P = .34).Among critically ill pediatric patients, the use of fresh red blood cells did not reduce the incidence of new or progressive multiple organ dysfunction syndrome (including mortality) compared with standard-issue red blood cells.ClinicalTrials.gov Identifier: NCT01977547.
View details for DOI 10.1001/jama.2019.17478
View details for PubMedID 31821429
Evaluating Mortality Risk Adjustment Among Children Receiving Extracorporeal Support for Respiratory Failure.
ASAIO journal (American Society for Artificial Internal Organs : 1992)
This study evaluates whether three commonly used pediatric intensive care unit (PICU) severity of illness scores, pediatric risk of mortality score (PRISM) III, pediatric index of mortality (PIM) 2, and pediatric logistic organ dysfunction (PELOD), are the appropriate tools to discriminate mortality risk in children receiving extracorporeal membrane oxygenation (ECMO) support for respiratory failure. This study also evaluates the ability of the Pediatric Risk Estimate Score for Children Using Extracorporeal Respiratory Support (Ped-RESCUERS) to discriminate mortality risk in the same population, and whether Ped-RESCUERS' discrimination of mortality is improved by additional clinical and laboratory measures of renal, hepatic, neurologic, and hematologic dysfunction. A multi-institutional retrospective cohort study was conducted on children aged 29 days to 17 years with respiratory failure requiring respiratory ECMO support. Discrimination of mortality was evaluated with the area under the receiver operating curve (AUC); model calibration was measured by the Hosmer-Lemeshow goodness of fit test and Brier score. Admission PRISM-III, PIM-2, and PELOD were found to have poor ability to discriminate mortality with an AUC of 0.56 [0.46-0.66], 0.53 [0.43-0.62], and 0.57 [0.47-0.67], respectively. Alternatively, Ped-RESCUERS performed better with an AUC of 0.68 [0.59-0.77]. Higher alanine aminotransferase, ratio of the arterial partial pressure of oxygen the fraction of inspired oxygen, and lactic acidosis were independently associated with mortality and, when added to Ped-RESCUERS, resulted in an AUC of 0.75 [0.66-0.82]. Admission PRISM-III, PIM-2, and PELOD should not be used for pre-ECMO risk adjustment because they do not discriminate death. Extracorporeal membrane oxygenation population-derived scores should be used to risk adjust ECMO populations as opposed to general PICU population-derived scores.
View details for PubMedID 29746311
Should Extracorporeal Membrane Oxygenation Be Offered? An International Survey
JOURNAL OF PEDIATRICS
2017; 182: 107-113
To assess the current attitudes of extracorporeal membrane oxygenation (ECMO) program directors regarding eligibility for ECMO among children with cardiopulmonary failure.Electronic cross-sectional survey of ECMO program directors at ECMO centers worldwide within the Extracorporeal Life Support Organization directory (October 2015-December 2015).Of 733 eligible respondents, 226 (31%) completed the survey, 65% of whom routinely cared for pediatric patients. There was wide variability in whether respondents would offer ECMO to any of the 5 scenario patients, ranging from 31% who would offer ECMO to a child with trisomy 18 to 76% who would offer ECMO to a child with prolonged cardiac arrest and indeterminate neurologic status. Even physicians practicing the same specialty sometimes held widely divergent opinions, with 50% of pediatric intensivists stating they would offer ECMO to a child with severe developmental delay and 50% stating they would not. Factors such as quality of life and neurologic status influenced decision making and were used to support decisions for and against offering ECMO.ECMO program directors vary widely in whether they would offer ECMO to various children with cardiopulmonary failure. This heterogeneity in physician decision making underscores the need for more evidence that could eventually inform interinstitutional guidelines regarding patient selection for ECMO.
View details for DOI 10.1016/j.jpeds.2016.12.025
View details for Web of Science ID 000396252400019
View details for PubMedID 28041665
The use of extracorporeal membrane oxygenation in pediatric patients with sickle cell disease
2013; 28 (5): 424-432
Previous reports have described the use of extracorporeal membrane oxygenation (ECMO) for acute chest syndrome of sickle cell disease (SCD). However, there have been no reports of venoarterial (VA) ECMO for cardiac dysfunction in patients with SCD. We describe a patient with SCD and life-threatening cardiogenic shock who was successfully treated with VA ECMO. Furthermore, SCD patients have unique comorbidities that warrant particular consideration when utilizing ECMO. We discuss these considerations and review the documented experience with ECMO for pediatric SCD patients from the Extracorporeal Life Support Organization (ELSO) registry. From 1990 until 2012, 52% of the 65 pediatric patients with SCD placed on ECMO survived, with 85% of those receiving venovenous (VV) ECMO surviving and 43% of those receiving VA ECMO surviving. However, significant complications, such as bleeding, neurological injury and kidney injury, also occurred with both VV and VA ECMO. Ten percent of SCD patients receiving VA ECMO experienced either a cerebral infarct or hemorrhage; our patient suffered a cerebrovascular accident while on ECMO, though she survived with good neurologic outcome. To our knowledge, this is the first report of a pediatric patient with SCD and cardiogenic shock successfully managed with VA ECMO. In conjunction with the ELSO registry review, this case report suggests that, while VA ECMO can be successfully used in patients with SCD and severe cardiovascular dysfunction, clinicians should also be aware of the potential for serious complications in this high-risk population.
View details for DOI 10.1177/0267659113485873
View details for Web of Science ID 000323311800009
View details for PubMedID 23630196
View details for PubMedCentralID PMC4414397
Gradient washout and secondary nephrogenic diabetes insipidus after brain injury in an infant: a case report.
Journal of medical case reports
2020; 14 (1): 183
BACKGROUND: Disorders of water and sodium balance can occur after brain injury. Prolonged polyuria resulting from central diabetes insipidus and cerebral salt wasting complicated by gradient washout and a type of secondary nephrogenic diabetes insipidus, however, has not been described previously, to the best of our knowledge. We report an unusual case of an infant with glioblastoma who, after tumor resection, was treated for concurrent central diabetes insipidus and cerebral salt wasting complicated by secondary nephrogenic diabetes insipidus.CASE PRESENTATION: A 5-month-old Hispanic girl was found to have a large, hemorrhagic, suprasellar glioblastoma causing obstructive hydrocephalus. Prior to mass resection, she developed central diabetes insipidus. Postoperatively, she continued to have central diabetes insipidus and concurrent cerebral salt wasting soon after. She was managed with a vasopressin infusion, sodium supplementation, fludrocortisone, and urine output replacements. Despite resolution of her other major medical issues, she remained in the pediatric intensive care unit for continual and aggressive management of water and sodium derangements. Starting on postoperative day 18, her polyuria began increasing dramatically and did not abate with increasing vasopressin. Nephrology was consulted. Her blood urea nitrogen was undetectable during this time, and it was thought that she may have developed a depletion of inner medullary urea and osmotic gradient: a "gradient washout." Supplemental dietary protein was added to her enteral nutrition, and her fluid intake was decreased. Within 4 days, her blood urea nitrogen increased, and her vasopressin and fluid replacement requirements significantly decreased. She was transitioned soon thereafter to subcutaneous desmopressin and transferred out of the pediatric intensive care unit.CONCLUSIONS: Gradient washout has not been widely reported in humans, although it has been observed in the mammalian kidneys after prolonged polyuria. Although not a problem with aquaporin protein expression or production, gradient washout causes a different type of secondary nephrogenic diabetes insipidus because the absence of a medullary gradient impairs water reabsorption. We report a case of an infant who developed complex water and sodium imbalances after brain injury. Prolonged polyuria resulting from both water and solute diuresis with low enteral protein intake was thought to cause a urea gradient washout and secondary nephrogenic diabetes insipidus. The restriction of fluid replacements and supplementation of enteral protein appeared adequate to restore the renal osmotic gradient and efficacy of vasopressin.
View details for DOI 10.1186/s13256-020-02536-0
View details for PubMedID 33036650
Burnout in Pediatric Critical Care Medicine Fellows.
Critical care medicine
OBJECTIVES: Assess the overall level of burnout in pediatric critical care medicine fellows and examine factors that may contribute to or protect against its development.DESIGN: Cross-sectional observational study.SETTING: Accreditation Council for Graduate Medical Education-accredited pediatric critical care medicine fellowship programs across the United States.SUBJECTS: Pediatric critical care medicine fellows and program directors.INTERVENTIONS: Web-based survey that assessed burnout via the Maslach Burnout Inventory, as well as other measures that elicited demographics, sleepiness, social support, perceptions about prior training, relationships with colleagues, and environmental burnout.MEASUREMENTS AND MAIN RESULTS: One-hundred eighty-seven fellows and 47 program directors participated. Fellows from 30% of programs were excluded due to lack of program director participation. Average values on each burnout domain for fellows were higher than published values for other medical professionals. Personal accomplishment was greater (lower burnout) among fellows more satisfied with their career choice (beta 9.319; p ≤ 0.0001), spiritual fellows (beta 1.651; p = 0.0286), those with a stress outlet (beta 3.981; p = 0.0226), those comfortable discussing educational topics with faculty (beta 3.078; p = 0.0197), and those comfortable seeking support from their co-fellows (beta 3.762; p = 0.0006). Depersonalization was higher for second year fellows (beta 2.034; p = 0.0482), those with less educational debt (beta -2.920; p = 0.0115), those neutral/dissatisfied with their career choice (beta -6.995; p = 0.0031), those with nursing conflict (beta -3.527; p = 0.0067), those who perceived burnout among co-fellows (beta 1.803; p = 0.0352), and those from ICUs with an increased number of patient beds (beta 5.729; p ≤ 0.0001). Emotional exhaustion was higher among women (beta 2.933; p = 0.0237), those neutral/dissatisfied with their career choice (beta -7.986; p = 0.0353), and those who perceived burnout among co-fellows (beta 5.698; p ≤ 0.0001). Greater sleepiness correlated with higher burnout by means of lower personal accomplishment (r = -1.64; p = 0.0255) and higher emotional exhaustion (r = 0.246; p = 0.0007). Except for tangible support, all other forms of social support showed a small to moderate correlation with lower burnout.CONCLUSIONS: Pediatric critical care medicine fellows in the United States are experiencing high levels of burnout, which appears to be influenced by demographics, fellow perceptions of their work environment, and satisfaction with career choice. The exclusion of fellows at 30% of the programs may have over or underestimated the actual level of burnout in these trainees.
View details for DOI 10.1097/CCM.0000000000004290
View details for PubMedID 32118699
Effects of ABO Matching of Platelet Transfusions in Critically Ill Children.
Pediatric critical care medicine : a journal of the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies
2019; 20 (2): e61–e69
To determine if transfusing ABO compatible platelets has a greater effect on incremental change in platelet count as compared to ABO incompatible platelets in critically ill children.Secondary analysis of a prospective, observational study. Transfusions were classified as either ABO compatible, major incompatibility, or minor incompatibility. The primary outcome was the incremental change in platelet count. Transfusion reactions were analyzed as a secondary outcome.Eighty-two PICUs in 16 countries.Children (3 d to 16 yr old) were enrolled if they received a platelet transfusion during one of the predefined screening weeks.None.Five-hundred three children were enrolled and had complete ABO information for both donor and recipient, as well as laboratory data. Three-hundred forty-two (68%) received ABO-identical platelets, 133 (26%) received platelets with major incompatibility, and 28 (6%) received platelets with minor incompatibility. Age, weight, proportion with mechanical ventilation or underlying oncologic diagnosis did not differ between the groups. After adjustment for transfusion dose, there was no difference in the incremental change in platelet count between the groups; the median (interquartile range) change for ABO-identical transfusions was 28 × 10 cells/L (8-68 × 10 cells/L), for transfusions with major incompatibility 26 × 10 cells/L (7-74 × 10 cells/L), and for transfusions with minor incompatibility 54 × 10 cells/L (14-81 × 10 cells/L) (p = 0.37). No differences in count increment between the groups were noted for bleeding (p = 0.92) and nonbleeding patients (p = 0.29). There were also no differences observed between the groups for any transfusion reaction (p = 0.07).No differences were seen in the incremental change in platelet count nor in transfusion reactions when comparing major ABO incompatible platelet transfusions with ABO compatible transfusions in a large study of critically ill children. Studies in larger, prospectively enrolled cohorts should be performed to validate whether ABO matching for platelet transfusions in critically ill children is necessary.
View details for DOI 10.1097/PCC.0000000000001779
View details for PubMedID 30422914
- Disseminated Intravascular Coagulation and Acute Liver Injury from Ethanol Embolization of an Arteriovenous Malformation. Journal of vascular and interventional radiology : JVIR 2018; 29 (3): 437–39
Platelet Transfusion Practices in Critically Ill Children.
Critical care medicine
2018; 46 (8): 1309–17
Little is known about platelet transfusions in pediatric critical illness. We sought to describe the epidemiology, indications, and outcomes of platelet transfusions among critically ill children.Prospective cohort study.Multicenter (82 PICUs), international (16 countries) from September 2016 to April 2017.Children ages 3 days to 16 years prescribed a platelet transfusion in the ICU during screening days.None.Over 6 weeks, 16,934 patients were eligible, and 559 received at least one platelet transfusion (prevalence, 3.3%). The indications for transfusion included prophylaxis (67%), minor bleeding (21%), and major bleeding (12%). Thirty-four percent of prophylactic platelet transfusions were prescribed when the platelet count was greater than or equal to 50 × 10 cells/L. The median (interquartile range) change in platelet count post transfusion was 48 × 10 cells/L (17-82 × 10 cells/L) for major bleeding, 42 × 10 cells/L (16-80 × 10 cells/L) for prophylactic transfusions to meet a defined threshold, 38 × 10 cells/L (17-72 × 10 cells/L) for minor bleeding, and 25 × 10 cells/L (10-47 × 10 cells/L) for prophylaxis in patients at risk of bleeding from a device. Overall ICU mortality was 25% but varied from 18% to 35% based on indication for transfusion. Upon adjusted analysis, total administered platelet dose was independently associated with increased ICU mortality (odds ratio for each additional 1 mL/kg platelets transfused, 1.002; 95% CI, 1.001-1.003; p = 0.005).The majority of platelet transfusions are given as prophylaxis to nonbleeding children, and significant variation in platelet thresholds exists. Studies are needed to clarify appropriate indications, with focus on prophylactic transfusions.
View details for DOI 10.1097/CCM.0000000000003192
View details for PubMedID 29727368
High-volume bilateral chylothorax presenting with hypoxemia and shock in a pediatric patient following tracheostomy revision: a case report.
Journal of medical case reports
2015; 9: 235-?
Chylothorax is a rare complication of surgical neck dissection. This is the first reported pediatric case of bilateral chylothorax following cervical surgery and the first to occur after tracheoplasty. Chylothorax can lead to significant complications, including hypoxemia and shock, and requires timely treatment. This case report discusses the clinical presentation, diagnosis, and treatment of our patient and reviews possible pathophysiologic mechanisms to explain the development of postoperative bilateral chylous effusions.An 18-month-old white baby girl with a complex past medical history including choanal atresia, atrioventricular septal defect, failure to thrive, developmental delay, and tracheostomy dependence developed significant hypoxemia and shock following a routine tracehostomy revision. She was subsequently found to have developed massive bilateral chylothorax, requiring escalation of mechanical ventilation, thoracostomy tube drainage, vasoactive support, and eventual surgical ligation of her thoracic duct.Massive bilateral chylothorax is a rare but potentially life-threatening complication following tracheoplasty. Clinicians caring for this patient population postoperatively should be aware of this potential complication and its management.
View details for DOI 10.1186/s13256-015-0721-6
View details for PubMedID 26493840
View details for PubMedCentralID PMC4618843
Refractory hypoxemia caused by hepatopulmonary syndrome: a case report.
Journal of medical case reports
2014; 8: 418-?
Hepatopulmonary syndrome is a clinical syndrome that can affect patients of all ages with liver disease and is more common in children with biliary atresia. Contrast echocardiography is the test of choice to diagnose the presence of intrapulmonary vascular dilatation. The established treatment for hepatopulmonary syndrome is liver transplantation.We present the case of an 8-month-old Caucasian baby boy with a history of biliary atresia, polysplenia, and interrupted inferior vena cava who presented with hypoxemia and cyanosis that progressed rapidly. A chest computed tomography angiogram revealed significant dilatation of the pulmonary vasculature, prompting further evaluation and diagnosis of hepatopulmonary syndrome with contrast echocardiography. He was maintained on a milrinone infusion while awaiting liver transplantation. His hypoxemia improved slowly following liver transplantation, requiring tracheostomy and prolonged ventilator dependence.Hepatopulmonary syndrome should be included in the differential for progressive hypoxemia in children with liver disease, particularly those with biliary atresia. Imaging with chest computed tomography angiogram and contrast echocardiography should be considered in cases of unexplained refractory hypoxemia.
View details for DOI 10.1186/1752-1947-8-418
View details for PubMedID 25491238
View details for PubMedCentralID PMC4295258
Early Outcomes After Extracardiac Conduit Fontan Operation Without Cardiopulmonary Bypass
2012; 33 (7): 1078-1085
Cardiopulmonary bypass is associated with a systemic inflammatory response. The authors hypothesized that avoiding cardiopulmonary bypass would lead to improved postoperative outcomes for patients undergoing the extracardiac Fontan operation, the final stage in surgical palliation of univentricular congenital heart defects. A review of the Children's Heart Center Database showed a total of 73 patients who underwent an initial Fontan operation at Lucile Packard Children's Hospital at Stanford between 1 November 2001 and 1 November 2006. These patients were divided into two groups: those who underwent cardiopulmonary bypass (n = 26) and those who avoided cardiopulmonary bypass (n = 47). Preoperative demographics, hemodynamics, and early postoperative outcomes were analyzed. The two groups had comparable preoperative demographic characteristics and hemodynamics except that the average weight of the off-bypass group was greater (17.9 ± 9.1 vs 14.2 ± 2.7 kg; P = 0.01). Intraoperatively, the off-bypass group trended toward a lower rate of Fontan fenestration (4.3 vs 19.2%; P = 0.09), had lower common atrial pressures (4.6 ± 1.4 vs 5.5 ± 1.5 mmHg; P = 0.05), and Fontan pressures (11.9 ± 2.1 vs 14.2 ± 2.4 mmHg; P ≤ 0.01), and required less blood product (59.1 ± 37.6 vs 91.9 ± 49.4 ml/kg; P ≤ 0.01). Postoperatively, there were no significant differences in hemodynamic parameters, postoperative colloid requirements, duration of mechanical ventilation, volume or duration of pleural drainage, or duration of cardiovascular intensive care unit or hospital stay. Avoiding cardiopulmonary bypass influenced intraoperative hemodynamics and the incidence of fenestration but did not have a significant impact on the early postoperative outcomes of children undergoing the Fontan procedure.
View details for DOI 10.1007/s00246-012-0228-5
View details for PubMedID 22349678